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Lichen planus (LP) is an idiopathic disorder that presents with cutaneous and genital manifestations. Esophageal LP (ELP) was first described by Al-Shihabi and Jackson [J Laryngol Otol 1982;96:567-571] in 1982. Only approximately 80 cases have been documented in the literature since. It is a rare and underrecognized disorder, leading to a delay in diagnosis and a lack of standardized management. We describe the presentation, diagnosis, and management of 6 cases of ELP, at a tertiary institution, because we believe that an increasing awareness of this condition can help identify more cases and increase our understanding of this interesting condition.

Lichen planus (LP) is a chronic idiopathic disorder involving the skin and mucosal surfaces. Although oral mucosal involvement is common, esophageal LP (ELP) is uncommon and also under-reported. Here, we present a case of ELP who was symptomatic with dysphagia for a year, but was untreated. Increasing awareness of this condition can help identify more cases and increase our understanding of this uncommon but interesting condition.

Papular mucinosis is an uncommon, idiopathic disorder characterized by dermal mucin deposition and increased collagen in the skin and internal organs. Its clinical presentation is characterized by dome-shaped, flesh colored papules that are closely spaced or linearly arranged. Papular mucinosis has been individually associated with several entities that include discoid lupus erythematosus, systemic lupus erythematosus and monoclonal gammopathy of undetermined significance. We encountered a 60-year-old woman with papular mucinosis in the setting of three concurrent disorders: discoid lupus erythematous, systemic lupus erythematosus and IgG paraproteinemia. Furthermore, we have reported the first case in North America of papular mucinosis being successfully treated with intralesional hyaluronidase.

Trachyonychia or rough nails, may present as an idiopathic disorder of the nails or it can be associated with other dermatological conditions. The dystrophic nail findings seen in trachyonychia are characterized by brittle, thin nails, with excessive longitudinal ridging. The most common histopathologic features associated with trachyonychia are spongiosis and exocytosis of inflammatory cells into the nail epithelia; typical features of lichen planus or psoriasis can also be detected. Determining the cause of trachyonychia is challenging. Treatment is often unsatisfactory, although in general it should be aimed at the underlying cause, if found. In most cases, the nail abnormalities improve spontaneously.

Eosinophilic cellulitis (EC) is a rare idiopathic disorder, first described as a "recurrent granulomatous dermatitis with eosinophilia", that mimics cellulitis of infectious origin. We describe here a previously healthy 11-year-old girl who experienced fever and tender erythematous patch lesions after trauma to her knees. Because of the relapsing cellulitis-like skin lesions, skin biopsies were taken, resulting in a diagnosis of EC. The patient responded well to oral prednisolone but experienced side effects and relapse during dose tapering. She was switched from prednisolone to cyclosporine. Her EC remained under control, and she showed no evidence of relapse after discontinuation of cyclosporine.

Parkinson’s disease is an idiopathic disorder of the central nervous system, characterized by resting tremors, muscular rigidity, slow and decreased movements. Oral rehabilitation of these patients requires special care, especially in those cases where the patient’s socioeconomic status is not good and patient cannot come several times for fabrication of a complete denture. This clinical report presents a case of a Parkinson’s patient who was completely rehabilitated in 3 appointments using special techniques. Border molding, final impression and jaw relation procedures were done in one appointment by using a custom tray with detachable handles and occlusal rims.

Apocrine chromhidrosis is a rare, chronic, idiopathic disorder, characterized by the excretion of pigmented sweat. A 26-year-old woman presented with a 3-year history of dark blue secretions on bilateral malar cheeks. On examination, upon pressure on the cheeks, a dark blue fluid was expressed, which appeared to arise primarily from the follicle. The patient had not appreciated it, but on examination of her axillae, a very subtle blue coloring was observed. Histopathologic examination revealed apocrine glands in the deep reticular dermis. Bluish cytoplasmic granules were observed in the apocrine epithelium lining, which correspond to lipofuscin granules. The diagnosis of apocrine cromhidrosis was made. We began treatment with 20 percent aluminum chloride hexahydrate solution and capsaicin cream with poor tolerance. Finally, we treated with botulinum toxin type A with a successful response. We report a case of facial and axillary apocrine cromhidrosis with good response to botulinum toxin type A.

Pulmonary alveolar microlithiasis (PAM) is a rare idiopathic disorder, usually with familial association, leading to alveolar deposition of calcium phosphate microliths. A 48-year-old female patient was referred to our clinic for bone scintigraphy because of severe osteoporosis (L1–L4 T score was −3.5 ; serum calcium values and 24-h calciuria in normal range). Bone scintigraphy showed bilateral, intense pulmonary 99mTc- methylenediphosphonate uptake, which led us to clinical suspicion of PAM. The chest radiograph revealed extensive, bilateral micronodular sand-like appearance, predominantly in the lower lung fields. The diagnosis of PAM was confirmed by transbronchial lung biopsy. Although the bone scintigraphy finding is sometimes negative in the early stage of PAM , this imaging method could be very useful in the detection of PAM.

A 94-year-old man presented with a relatively asymptomatic eruption of his forearms, abdomen, and back comprising symmetric erythematous papules and plaques. Generalized granuloma annulare is an idiopathic disorder that occurs more commonly in older patients and is characterized by numerous flesh-colored-to-erythematous papules. It is distinguished histologically by palisading granulomas with central connective tissue degeneration and with central mucin. Therapy has been attempted with various modalities with moderate efficacy.

金沢大学医薬保健研究域医学系, Background: Lymphangioleiomyomatosis (LAM) is a rare idiopathic disorder that occurs in women of childbearing age, and consists of a diffuse proliferation of abnormal smooth muscle cells along the thoracic and abdominal lymphogenous route. Case presentation: We experienced a case of a 47-yo woman with recent history of systemic lupus erythematosus (SLE) diagnosed with endometrial cancer, initially suspected to have metastasized to pelvic and para-aortic lymph nodes based on preoperative diagnostic imaging. Subsequent pathological diagnosis revealed stage IB endometrial cancer without evidence of lymph node involvement. Instead, enlarged pelvic and para-aortic lymph nodes were found to be due to extrapulmonary LAM, from a primary lesion found inside the uterine myometrium. SLE improved after surgery. Conclusion: This is the first reported case of comorbid endometrial cancer, SLE, and aggressive LAM metastasizing to regional lymph nodes, and strengthens the clinical evidence for a common role of mTOR pathway hyperactivity and estrogen responsiveness in the pathophysiology of metastasizing lesions of the genital tract. © 2016 The Author(s).